Guillain-Barré syndrome in patients with Charcot-Marie-Tooth type 1A disease, probably a non-random association

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY

Neurophysiologie Clinique/Clinical Neurophysiology Pub Date : 2025-03-21 DOI:10.1016/j.neucli.2025.103071

Jean-Baptiste Davion , Jérôme Devaux , Céline Tard , Armelle Magot , François Cassim , Yann Péréon

{"title":"Guillain-Barré syndrome in patients with Charcot-Marie-Tooth type 1A disease, probably a non-random association","authors":"Jean-Baptiste Davion , Jérôme Devaux , Céline Tard , Armelle Magot , François Cassim , Yann Péréon","doi":"10.1016/j.neucli.2025.103071","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><div>To describe four cases of Charcot-Marie-Tooth disease type 1A (CMT1A) who developed Guillain-Barré syndrome (GBS), respectively the most frequent genetic and inflammatory neuropathies.</div></div><div><h3>Methods</h3><div>We described the patients’ clinical and electrodiagnostic characteristics.</div></div><div><h3>Results</h3><div>Our CMT1A patients developed typical GBS at various ages (3 to 76 years). GBS-related clinical manifestations were different within patients, with various severity degrees of seve (weakness, respiratory failure). Nerve conduction studies revealed more severe demyelinating features than expected in patients with no CMT1A. High cerebrospinal fluid protein level was found in 3 patients. GBS outcome was mainly good, although some patients only slowly improved.</div></div><div><h3>Conclusions</h3><div>Our cases are close to the previously described cases of acute worsening in CMT1A, and presented with many electrophysiological features of GBS. Overall, GBS prognosis does not seem worse in CMT1A patients than in other patients. If GBS and CMT1A were independent, the expected frequency of co-occurrence of GBS and CMT1A in our two French regions should be 1 case every 137 years. As we observed 4 cases in only 5 years, we suspect that CMT1A is a risk factor of GBS.</div></div><div><h3>Significance</h3><div>These cases bring further evidence for a non-random link between inflammatory and genetic neuropathies.</div></div>","PeriodicalId":19134,"journal":{"name":"Neurophysiologie Clinique/Clinical Neurophysiology","volume":"55 4","pages":"Article 103071"},"PeriodicalIF":2.7000,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neurophysiologie Clinique/Clinical Neurophysiology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0987705325000309","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Objective

To describe four cases of Charcot-Marie-Tooth disease type 1A (CMT1A) who developed Guillain-Barré syndrome (GBS), respectively the most frequent genetic and inflammatory neuropathies.

Methods

We described the patients’ clinical and electrodiagnostic characteristics.

Results

Our CMT1A patients developed typical GBS at various ages (3 to 76 years). GBS-related clinical manifestations were different within patients, with various severity degrees of seve (weakness, respiratory failure). Nerve conduction studies revealed more severe demyelinating features than expected in patients with no CMT1A. High cerebrospinal fluid protein level was found in 3 patients. GBS outcome was mainly good, although some patients only slowly improved.

Conclusions

Our cases are close to the previously described cases of acute worsening in CMT1A, and presented with many electrophysiological features of GBS. Overall, GBS prognosis does not seem worse in CMT1A patients than in other patients. If GBS and CMT1A were independent, the expected frequency of co-occurrence of GBS and CMT1A in our two French regions should be 1 case every 137 years. As we observed 4 cases in only 5 years, we suspect that CMT1A is a risk factor of GBS.

Significance

These cases bring further evidence for a non-random link between inflammatory and genetic neuropathies.

查看原文本刊更多论文

1A型Charcot-Marie-Tooth病患者的格林-巴罗综合征，可能是非随机关联

目的分析4例1A型沙科-玛丽-图斯病（CMT1A）并发吉兰-巴罗综合征（GBS）的病例，分别为最常见的遗传性和炎症性神经病变。方法对患者的临床和电诊断特点进行描述。结果我们的CMT1A患者在不同年龄（3 - 76岁）出现了典型的GBS。患者的gbs相关临床表现不同，严重程度不同（虚弱、呼吸衰竭）。神经传导研究显示，在没有CMT1A的患者中，脱髓鞘特征比预期的更严重。3例脑脊液蛋白水平升高。GBS结果主要是良好的，尽管一些患者只有缓慢的改善。结论sour病例与先前描述的CMT1A急性加重病例接近，并表现出GBS的许多电生理特征。总体而言，CMT1A患者的GBS预后似乎并不比其他患者差。如果GBS和CMT1A是独立的，那么我们的两个法国地区GBS和CMT1A同时出现的预期频率应为每137年1例。由于我们在短短5年内观察到4例病例，我们怀疑CMT1A是GBS的一个危险因素。这些病例进一步证明了炎症性神经病变与遗传性神经病变之间存在非随机联系。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Neurophysiologie Clinique/Clinical Neurophysiology 医学-临床神经学

CiteScore

5.20

自引率

3.30%

发文量

审稿时长

60 days

期刊介绍： Neurophysiologie Clinique / Clinical Neurophysiology (NCCN) is the official organ of the French Society of Clinical Neurophysiology (SNCLF). This journal is published 6 times a year, and is aimed at an international readership, with articles written in English. These can take the form of original research papers, comprehensive review articles, viewpoints, short communications, technical notes, editorials or letters to the Editor. The theme is the neurophysiological investigation of central or peripheral nervous system or muscle in healthy humans or patients. The journal focuses on key areas of clinical neurophysiology: electro- or magneto-encephalography, evoked potentials of all modalities, electroneuromyography, sleep, pain, posture, balance, motor control, autonomic nervous system, cognition, invasive and non-invasive neuromodulation, signal processing, bio-engineering, functional imaging.